title: A genetic defect of granulocyte oxidative metabolism in a man with disseminated aspergillosis.
abstract: A 26-year-old man presented with progressive pneumonia, and Aspergillus was grown from cultures of lung, cutaneous nodules, and urine. His PMNs had a poor CL response after exposure to phagocytic stimuli (S. aureus, latex, aggregated IgG and IgG-coated latex) (p less than 0.01 vs. controls) and soluble stimuli (PMA, sodium fluoride, and Con A) (p less than 0.05). His PMNs failed to reduce NBT, oxidize 14C-1 glucose (p less than 0.001), or iodinate proteins (p less than 0.001), normally, compared with controls, and his PMNs killed Candida albicans and Staphylococcus aureus abnormally (p less than 0.05). The patient was anergic; his plasma inhibited responsiveness of his lymphocytes to stimulation with Aspergillus and Candida antigens. His lymphocytes failed to produce the lymphokine LMIF normally. The patient's 10-month-old daughter was demonstrated to have the same defects of PMN metabolism and function. The findings in this patient were similar to those in CGd, but transmission of the defect from father to daughter and the presence of lymphocyte abnormalities make this diagnosis unlikely. Inhalation of Aspergillus by patients with defective PMN oxidative metabolism may be associated with development of significant infection.
mesh_terms: D000328:Adult; D001229:Aspergillosis, Allergic Bronchopulmonary / Q000235:genetics*; D001230:Aspergillus / Q000276:immunology; D001770:Blood Bactericidal Activity; D002176:Candida albicans; D013293:Enterococcus faecalis; D006098:Granulocytes / Q000378:metabolism*; D006801:Humans; D007961:Leukocyte Migration-Inhibitory Factors / Q000096:biosynthesis; D008163:Luminescent Measurements; D008213:Lymphocyte Activation; D008297:Male; D009504:Neutrophils / Q000378:metabolism; D009580:Nitroblue Tetrazolium; D010587:Phagocytosis; D013211:Staphylococcus aureus
chemical_list: D007961:Leukocyte Migration-Inhibitory Factors; D009580:Nitroblue Tetrazolium